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Restrictive pulmonary dysfunction and its predictors in young patients with β-thalassaemia major.
MedLine Citation:
PMID:  22328228     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
BACKGROUND: Pulmonary dysfunction represents one of the most undervalued and less recognized complications in patients with β-thalassaemia. OBJECTIVES: The aim of this study was to assess the pattern of pulmonary dysfunction and consequently to investigate possible associated factors that might contribute to lung impairment in young patients with β-thalassaemia major. METHODS: Fifty-two children and young adults (mean age: 21.33 ± 6.24 years) with β-thalassaemia major on conventional treatment (transfusions and iron chelation therapy) were included in the study. A complete computerized pulmonary function testing (PFT) system for recording pulmonary diffusion capacity and simultaneous determination of alveolar volume and pulmonary volumes was equipped. RESULTS: Results showed that 20 patients (38.46%) had restrictive pulmonary pattern that was preferentially observed in older and shorter patients. Serum ferritin levels were higher in the restrictive group (2,096 ± 1,831 ng/dl) compared to patients with normal pulmonary function (1,354 ± 942 ng/dl) (P = 0.066). Diffusional impairment characterized by significantly lower DLCO*% values, was observed in the restrictive group (P = 0.004), implicating the 62.5% of the population studied. Paired linear correlations showed that age was negatively correlated to DLCO*% (r = -0.548, P < 0.001) and SaO(2) % (r = -0.789, P < 0.001) and with most of the pulmonary functional parameters that determine a restrictive. Multivariate regression analysis identified age as the major predictor for restrictive pulmonopathy followed by serum ferritin levels. CONCLUSIONS: Our study shows that pulmonary impairment is shown in a great proportion even among asymptomatic young thalassaemic patients, thus, regular screening of pulmonary function should be adopted in the routine clinical follow up of these patients. Pediatr Pulmonol. © 2012 Wiley Periodicals, Inc.
Authors:
Evangelia Bourli; Meropi Dimitriadou; Marina Economou; Efthimia Vlachaki; Athanasios Christoforidis; Eleni Maratou; Ioannis Stanopoulos; Paraskevi Argyropoulou; Victor Aivazis
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-2-10
Journal Detail:
Title:  Pediatric pulmonology     Volume:  -     ISSN:  1099-0496     ISO Abbreviation:  -     Publication Date:  2012 Feb 
Date Detail:
Created Date:  2012-2-13     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8510590     Medline TA:  Pediatr Pulmonol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2012 Wiley Periodicals, Inc.
Affiliation:
First Paediatric Department, Aristotle University of Thessaloniki, Thessaloniki, Greece.
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